We administered therapeutic doses of valproic acid (VPA), carbamazepine (CBZ), phenytoin (PHT), and phenobarbital (PHB) to mice for 7 days, and 8 hours after the final dose we measured the concentrations of carnitine in serum, liver, kidney, skeletal muscle, and heart, and in the 7 days' accumulated urine. The results for serum and urine show that VPA induced a significant increase in renal clearance of acylcarnitine without affecting that of free carnitine, whereas CBZ, PHT, and PHB significant increased clearance of free carnitine but not that of acylcarnitine. Thus, VPA appears to reduce tubular resorption of acylcarnitine, and CBZ, PHT, and PHB appear to reduce tubular resorption of free carnitine.
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http://dx.doi.org/10.1212/wnl.41.9.1444 | DOI Listing |
Metab Brain Dis
January 2025
Programa de Pós-Graduação em Ciências Farmacêuticas, Universidade Federal do Rio Grande do Sul, Avenida Ipiranga, 2752, Porto Alegre, CEP 90610-000, RS, Brazil.
Phenylketonuria is a genetic disorder characterized by high phenylalanine levels, the main toxic metabolite of the disease. Hyperphenylalaninemia can cause neurological impairment. In order to avoid this symptomatology, patients typically follow a phenylalanine-free diet supplemented with a synthetic formula that provides essential amino acids, including L-carnitine.
View Article and Find Full Text PDFHypertens Res
January 2025
Department of Cardiology, Kurume University Medical Center, 155-1 Kokubu-machi, Kurume, Fukuoka, 839-0863, Japan.
The ratio of serum acylcarnitines to free carnitines (AC/FC) reflects impaired cardiomyocyte β-oxidization. The effect of heart failure (HF) treatment on AC/FC remained unclear. This pilot study retrospectively examined treatment-induced AC/FC changes in patients with HF with preserved ejection fraction (HFpEF) and HF with reduced ejection fraction (HFrEF) in 148 consecutive inpatients undergoing echocardiography and carnitine measurement at admission and discharge.
View Article and Find Full Text PDFOrphanet J Rare Dis
January 2025
Laboratory of Metabolic Diseases, Department of Laboratory Medicine, University Medical Center Groningen, University of Groningen, Hanzeplein 1, Postbus, Groningen, 30001 - 9700 RB, the Netherlands.
Background: Glycogen storage disease (GSD) Ia is an ultra-rare inherited disorder of carbohydrate metabolism. Patients often present in the first months of life with fasting hypoketotic hypoglycemia and hepatomegaly. The diagnosis of GSD Ia relies on a combination of different biomarkers, mostly routine clinical chemical markers and subsequent genetic confirmation.
View Article and Find Full Text PDFMol Psychiatry
January 2025
Department of Psychiatry, New York University Grossman School of Medicine, New York, NY, USA.
A major challenge in the development of more effective therapeutic strategies for Alzheimer's disease (AD) is the identification of molecular mechanisms linked to specific pathophysiological features of the disease. Importantly AD has a two-fold higher incidence in women than men and a protracted prodromal phase characterized by amnestic mild-cognitive impairment (aMCI) suggesting that biological processes occurring early can initiate vulnerability to AD. Here, we used a sample of 125 subjects from two independent study cohorts to determine the levels in plasma (the most accessible specimen) of two essential mitochondrial markers acetyl-L-carnitine (LAC) and its derivative free-carnitine motivated by a mechanistic model in rodents in which targeting mitochondrial metabolism of LAC leads to the amelioration of cognitive function and boosts epigenetic mechanisms of gene expression.
View Article and Find Full Text PDFFood Sci Biotechnol
January 2025
Institute of Food Industrialization, Institutes of Green Bioscience and Technology, Seoul National University, Pyeongchang-gun, Gangwon-do 25354 Republic of Korea.
Sorghum () is a gluten-free supercrop with a high content of phenolic compounds, along with anti-nutrient factors such as tannin that limit its use in food. In this study, we conducted solid-state fermentation for sorghum with to reduce the tannin content and value-added sorghum by enhancing biological properties. The results showed that fermented sorghum had 1.
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